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Symbol ADAM22 contributors: npt/mct - updated : 10-05-2017
HGNC name ADAM metallopeptidase domain 22
HGNC id 201
Corresponding disease
EIEECA encephalopathy with epilepsy and cortical atrophy
Location 7q21.12      Physical location : 87.563.701 - 87.826.447
Synonym name
  • metalloproteinase-like,disintegrin-like,cysteine rich protein-like 1
  • a disintegrin and metalloproteinase domain 22
  • Synonym symbol(s) MDCL1, MDC2, MGC149832
    TYPE functioning gene
    SPECIAL FEATURE arranged in tandem
    STRUCTURE 268.75 kb     32 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Physical map
    HMG17P1 7q11-q21 high-mobility group (nonhistone chromosomal) protein 17 pseudogene 1 LOC285984 7q21.12 similar to Heterogeneous nuclear ribonucleoprotein A1 (Helix-destabilizing protein) (Single-strand binding protein) (hnRNP core protein A1) (HDP-1) (Topoisomerase-inhibitor suppressed) SEMA3D 7q21.12 sema domain, immunoglobulin domain (Ig), short basic domain, secreted, (semaphorin) 3D LOC392067 7 similar to dynein, cytoplasmic, light peptide GRM3 7q21.1-q21.2 glutamate receptor, metabotropic 3 FLJ31340 7q21.13 hypothetical protein FLJ31340 DMTF1 7q21.1 cyclin D binding myb-like transcription factor 1 C7orf23 7q21.1-q21.2 chromosome 7 open reading frame 23 TP53AP1 7q21.1 TP53 activated protein 1 CROT 7q21.1 carnitine O-octanoyltransferase ABCB4 7q21.1 ATP-binding cassette, sub-family B (MDR/TAP), member 4 ABCB1 7q21.1 ATP-binding cassette, sub-family B (MDR/TAP), member 1 RPIB9 7q21.13 Rap2-binding protein 9 MCFP 7q21.13 mitochondrial carrier family protein ASK 7q21.3 mitochondrial carrier family protein ADAM22 7q21.1 a disintegrin and metalloproteinase domain 22 SRI 7q21.1 sorcin FLJ23153 7q21.13 likely ortholog of mouse tumor necrosis-alpha-induced adipose-related protein LOC392068 7 similar to CSRP2 binding protein isoform a; CRP2 binding protein; cysteine rich protein 2 binding protein; CRP2 binding partner LOC392069 7 similar to Importin alpha-2 subunit (Karyopherin alpha-2 subunit) (SRP1-alpha) (RAG cohort protein 1) LOC219558 7q21.13 similar to elongation factor 1 alpha FLJ32110 7q21.13-q21.2 hypothetical protein FLJ32110 MGC26647 7q21.13 hypothetical protein MGC26647 STEAP 7q21 six transmembrane epithelial antigen of the prostate STEAP2 7q21 six transmembrane epithelial antigen of prostate 2 FLJ21062 7q21.2 hypothetical protein FLJ21062 LOC85865 7q21.2 hypothetical protein BC004923 CLDN12 7q21 claudin 12 PFTK1 7q21-q13 PFTAIRE protein kinase 1
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    31 - 3445 100 906 normal brain, not expressed in high grade glioma 2003 12589811
    isoform epsilon 1
    32 splicing 3393 - 899 - 2003 12589811
  • isoform delta 2
  • lacking exon 2
  • 30 splicing 3347 - 870 most abundant transcript in glioma 2003 12589811
  • isoform beta 4
  • lacking exon 2
  • 29 splicing 2744 - 859 not expressed in glioma 2003 12589811
    isoform beta 4
    28 splicing 2636 - 823 - 2003 12589811
  • isoform 5
  • lacking exon 2
  • 32 - 9623 - 963 absent in normal brain, only in glioma 2003 12589811
    lacking exon 2
    32 - 9626 - 964 not expressed in glioma 2003 12589811
    34 - - - - overexpressed inglioma 2003 12589811
    31 - 9539 - 935 low expressed in normal brain, overexpressed in glioma 2003 12589811
    lacking exon 2
    29 - 9220 - 890 expressed in one glioma specimen only 2003 12589811
    38 - - - - - 2003 12589811
    35 - - - - - 2003 12589811
    34 - - - - - 2003 12589811
    30 - 9341 - 869 - 2003 12589811
    Type restricted
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrineneuroendocrinepituitary  highly
    Nervousbrainlimbic systemhippocampus highly
     brainhindbraincerebellum highly
     nervecranial nerve   
     spinal cord   highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectivebone  highly
    SystemCellPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
    physiological period fetal
  • domains similar to hemorrhagic snake venom proteins (HSVP)
  • a disrupted Zn-binding site metalloproteinase
  • a disintegrin-like domains
  • a cysteine-rich domain
  • an EGF-like repeat
  • a transmembrane domain and a cytoplasmic tail
  • a peptidase M12B domain
  • extracellular domain interacting with LGI1, whereas its cytoplasmic PDZ-binding motif recruits DLG4 (Liu 2009)
  • ectodomain comprising four domains assembled together like a four-leaf clover, and each leaf represents one of the four domains including :
  • the metalloproteinase-like domain (domain M, residues 233435)
  • the disintegrin domain (domain D, residues 445529)
  • the cysteine-rich domain (domain C, residues 530676)
  • the epidermal growth factor-like domain (domain E, residues 677718) (Liu 2009)
  • conjugated GlycoP , MetalloP
    isoforms Precursor the propeptide of ADAM22 is essential for ADAM22 secretion and may play an intramolecular chaperone function, similar to the cases of ADAM17 and ADAM12
  • ADAM (a disintegrin and metalloprotease domain) family
  • CATEGORY adhesion , enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
    text anchored to the postsynaptic density by cytoskeletal scaffolds containing stargazin
    basic FUNCTION
  • involved in cell-cell and cell-matrix interactions integrin ligand in the brain
  • brain-specific cell surface protein, mediates growth inhibition using an integrin dependent pathway
  • non-catalytic ADAMs, recently identified to serve as the postsynaptic receptor for the secreted neurotransmission modulator LGI1 at neural synapses (Liu 2009)
  • by finely regulating the synaptic AMPA receptors, the LGI1-ADAM22 interaction maintains likely physiological brain excitability throughout life
  • CELLULAR PROCESS protein, degradation
    text neurogenesis
    a component
  • form a tertiary complex with postsynaptic density-95 (DLG4), a major scaffolding protein localized to the postsynaptic density of brain synapse (Liu 2009)
  • LGI1 and ADAM22 form an essential synaptic organizing complex that coordinates the maturation of excitatory synapses by regulating the functional incorporation of DLG4
    small molecule metal binding,
  • Zn2+
  • three putative calcium ions bound to ADAM22
  • protein
  • probable ligand for integrin in the brain
  • coupling to LGI1, enhancing AMPA receptor-mediated synaptic transmission in hippocampal slices
  • interacting with LGI1 (LGI1acts as a ligand that selectively binds to the postsynaptic receptor ADAM22, thereby regulating the glutamate-AMPA neurotransmission)
  • interaction with YWHAZ (can regulate cell adhesion and spreading, therefore it has a potential role in neural development and function)
  • with ADAM11 and ADAM23, binds LGI1 and LGI4 (LGI-ADAM system seems to be regulated not only by the affinity but also by the cell-type-specific expression of each protein) (Sagane 2008)
  • critical roles of a ligand-receptor complex, LGI1-ADAM22, in synaptic transmission and brain function
  • is a ligand secreted by Schwann cells that regulates peripheral nerve myelination via its cognate receptor ADAM22 expressed by neurons
  • cell & other
    corresponding disease(s) EIEECA
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --low  
    high-grade but not low-grade gliomas
    Variant & Polymorphism
    Candidate gene
    Therapy target